ABSTRACT
INTRODUCTION: Colchicine is a narrow therapeutic margin drug that does not have the adverse effects of corticosteroids and immunosuppressants. Its use in non-severe ocular inflammatory disease excluding Behcet's disease has not been studied. METHODS: We included patients seen in the internal medicine department of Dijon University Hospital consecutively between September 2020 and September 2021 if they had received colchicine during their pathology. Patients with suspected Behçet's disease were excluded. Treatment efficacy was studied in patients with at least one year of disease progression who had received more than one year of colchicine. Successful treatment was defined as a 50 % reduction in the number of annual relapses on colchicine. RESULTS: Sixteen patients were included (9 women and 7 men). They had recurrent anterior uveitis (n=10), recurrent scleritis (n=5) and intermediate uveitis. Opthalmological involvement was neither severe nor complicated. All patients combined, the annual relapse ratio (ARR) decreased from 1.8 (0.8-3.5) to 0.3 (0-1.6), (P=0.06). Colchicine was considered effective in three of 10 analyzable patients. In only one patient, treatment was stopped for adverse effects after six weeks. CONCLUSION: In view of the interesting benefit-risk ratio of colchicine, it seems appropriate to focus on this molecule in non-granulomatous anterior uveitis and non-severe recurrent scleritis.
Subject(s)
Behcet Syndrome , Scleritis , Uveitis, Anterior , Uveitis , Male , Humans , Female , Behcet Syndrome/complications , Behcet Syndrome/diagnosis , Behcet Syndrome/drug therapy , Colchicine/adverse effects , Scleritis/diagnosis , Scleritis/drug therapy , Scleritis/etiology , Vision Disorders , Uveitis, Anterior/complications , Uveitis, Anterior/drug therapy , Recurrence , Inflammation/complications , Uveitis/diagnosis , Uveitis/drug therapy , Uveitis/etiologyABSTRACT
BACKGROUND: Behçet's disease (BD) is a recurrent multisystemic disease responsible for occlusive vasculitis with arterial, venous and capillary involvement. The aim of this study was to determine the frequency and the features associated with the use of biotherapy in the management of patients followed in our department for BD. METHODS: This is a retrospective study of patients medical records followed for BD in a department of internal medicine from January 2005 to August 2018. RESULTS: A total of 41 patients were included with a mean age at diagnosis of 42.5±12.1 years (range 16 to 63) and a sex ratio men/women of 1.05. Oral and/or genital aphtosis was present in 70.7% of the patients. Other lesions were: ocular (78.0%), articular (46.3%), cutaneous (41.5%), central neurological (34.1%), vascular (26.8%), digestive (7.3%), pericardial (2.4%) and epididymal (2.4%). A biotherapy, interferon α and monoclonal antibodies, was used in 15 patients (36.6%), after failure of conventional treatments. The monoclonal antibodies were anti-TNFα (infliximab, adalimumab, certolizumab and golimumab) except in one patient for whom ustekinumab was used. Biotherapy was used in 46.9% of the patients with ocular involvement and never used in those patients without ocular involvement (P=0.01). CONCLUSION: Biotherapy is effective and represents a solution to the failures of conventional treatments in severe forms of Behçet's disease with ocular involvement.
Subject(s)
Behcet Syndrome/drug therapy , Biological Therapy , Adolescent , Adult , Biological Therapy/statistics & numerical data , Female , Hospital Departments , Humans , Internal Medicine , Male , Middle Aged , Retrospective Studies , Young AdultSubject(s)
Panniculitis/diagnosis , Panniculitis/etiology , Sarcoidosis/diagnosis , Aged , Female , HumansABSTRACT
Behçet's disease is a rare multisystemic vasculitis with an etiology that is still unknown. Neurological manifestations may be seen in approximately 5-15% of patients, and both parenchymal and extraparenchymal neurological involvement has been described. When cerebral venous thrombosis (CVT) is the main extraparenchymal manifestation of Behçet's disease, the condition is then dubbed "angio-Behçet's syndrome". However, arterial involvement is extremely rare, with only one reported case of vasculo-neuro-Behçet's disease, characterized by both venous and intracranial arterial involvement - until now. This report is of two patients diagnosed with Behçet's disease characterized by the concomitant presence of both cerebral arterial manifestations and CVT.
Subject(s)
Behcet Syndrome/physiopathology , Cerebral Arteries/physiopathology , Cerebral Veins/physiopathology , Adult , Behcet Syndrome/drug therapy , Cerebral Angiography , Female , Fibrinolytic Agents/therapeutic use , Humans , Intracranial Thrombosis/drug therapy , Intracranial Thrombosis/physiopathology , Magnetic Resonance Angiography , Magnetic Resonance Imaging , Male , Treatment Outcome , Venous Thrombosis/drug therapy , Venous Thrombosis/physiopathology , Young AdultABSTRACT
Hyper-IgE may be found under many pathological conditions. The role of IgE is essentially associated with the occurrence of allergic manifestations, which may be accompanied by an increase of their serum levels. Elevation of total IgE has also been reported in association with certain rare genetic immune deficiencies called hyper-IgE syndromes. Other circumstances such as infectious diseases, tumors or autoimmune diseases may also be accompanied by an excessive synthesis of IgE. Considering the diversity of these situations, discussion of the prognostic value of total IgE is useful to the internist.
Subject(s)
Immunoglobulin E/blood , Job Syndrome/diagnosis , Cytokines/blood , Humans , Internal Medicine , Job Syndrome/therapyABSTRACT
Systemic lupus erythematosus (SLE) is a chronic disease that considerably hampers patient's daily living. Qualitative studies with patients' interviews have been conducted to describe the experiences and perspectives of adults living with SLE. Among existing generic and disease-specific quality of life (QOL) questionnaires, none succeeded to exhaustively measure patient's preoccupations. However, these tools are useful to quantify the burden of the disease. Social precariousness, socioeconomic status and education level are intimately correlated to QOL measures, either generic or disease-specific. Musculoskeletal disease activity is also associated with a lower QOL. Using disease-specific tools may be useful because of a better aptitude to record an improvement in health status. Moreover, using generic and disease-specific questionnaires together may help to identify factors associated with a lower quality of life but not related to SLE from the patient's perspective (such as smoking or obesity). Developing new ways of recording QOL data in the future may help to evaluate the real benefit of using QOL scales in daily practice.
Subject(s)
Lupus Erythematosus, Systemic/psychology , Patient Reported Outcome Measures , Quality of Life , Adult , Humans , Risk Factors , Surveys and QuestionnairesABSTRACT
INTRODUCTION: Cat scratch disease is a pleiomorphic condition, sometimes with isolated ophthalmic involvement. We report the clinical observations of seven cases with ophthalmologic manifestations of cat scratch disease. OBSERVATIONS: There were seven patients, with a median age of 52 years, of whom five were women and three had unilateral involvement. Six exhibited Leber's stellate neuroretinitis, an incomplete syndrome in two cases, and one associated with chorioretinal foci. One patient had isolated retinal infiltrates. The diagnosis of cat scratch disease was confirmed by Bartonella henselae serology, positive in all cases. All patients received treatment with doxycycline. Ocular complications (with optic atrophy and macular retinal pigment epithelial changes) were noted in five cases. DISCUSSION: Ocular bartonellosis is an atypical clinical form. It requires a directed ancillary work-up with serology or PCR, which has the peculiarity of being highly specific if not very sensitive. Treatment is above all preventive. Antibiotics may be initiated. CONCLUSION: Cat scratch disease must be excluded in the work-up of posterior uveitis.
Subject(s)
Cat-Scratch Disease/diagnosis , Eye Infections, Bacterial/diagnosis , Retinitis/diagnosis , Adult , Aged , Animals , Bartonella henselae/isolation & purification , Cat-Scratch Disease/complications , Cats , Cohort Studies , Eye Infections, Bacterial/microbiology , Female , Humans , Male , Middle Aged , Papilledema/diagnosis , Papilledema/microbiology , Papilledema/pathology , Retinitis/microbiologyABSTRACT
We investigated the positivity rate, the detection rates for non-covered pathogens and the therapeutic impact of microbiological samples (MS) in community-acquired pneumonia (CAP), nursing home-acquired pneumonia (NHAP) and hospital-acquired pneumonia (HAP) in elderly hospitalised patients. Patients aged 75 years and over with pneumonia and hospitalised between 1/1/2013 and 30/6/2013 in the departments of medicine (5) and intensive care (1) of our university hospital were included. Microbiological findings, intra-hospital mortality and one-year mortality were recorded. Among the 217 patients included, there were 138 CAP, 56 NHAP and 23 HAP. MS were performed in 89.9, 91.1 and 95.6 % of CAP, NHAP and HAP, respectively. Microbiological diagnosis was made for 29, 11.8 and 27.3 % of patients for CAP, NHAP and HAP, respectively (p = 0.05). Non-covered pathogens were detected for 8 % of CAP, 2 % of NHAP and 13.6 % of HAP (p = 0.1). The antimicrobial spectrum was significantly more frequently reduced when the MS were positive (46.7 % vs. 10.8 % when MS were negative, p = 10(-7)). The MS positivity rate was significantly lower in NHAP than in CAP and HAP. MS revealed non-covered pathogens in only 2 % of NHAP. These results show the poor efficiency and weak clinical impact of MS in the management of pneumonia in hospitalised older patients and suggest that their use should be rationalised.
Subject(s)
Community-Acquired Infections/diagnosis , Community-Acquired Infections/microbiology , Cross Infection/diagnosis , Cross Infection/microbiology , Pneumonia/diagnosis , Pneumonia/microbiology , Age Factors , Aged , Aged, 80 and over , Anti-Infective Agents/therapeutic use , Community-Acquired Infections/mortality , Community-Acquired Infections/therapy , Cross Infection/mortality , Cross Infection/therapy , Disease Management , Female , Hospital Mortality , Hospitalization , Hospitals , Humans , Male , Nursing Homes , Pneumonia/mortality , Pneumonia/therapy , Retrospective Studies , Severity of Illness Index , Treatment OutcomeABSTRACT
Birt-Hogg-Dubé (SBHD) syndrome is a rare autosomal dominant genodermatosis, predisposing to the occurrence of tumors associated with skin manifestations. We report a male patient who presented with a history of recurrent pneumothorax and was treated by nephrectomy for a left kidney carcinoma at 20 years old. Skin examination disclosed numerous fibrofolliculomas of the scalp. During follow-up, surgical resection of parotid oncocytomas and of a parathyroid adenoma were performed. SBHD was confirmed by molecular biology. In the presence of fibrofolliculomas and kidney tumors and recurrent spontaneous pneumothorax, a diagnosis of SHBD syndrome should be discussed, and tests need to be performed on the whole family.
Subject(s)
Birt-Hogg-Dube Syndrome , Neoplasm Recurrence, Local , Neoplasms, Multiple Primary , Skin Neoplasms , Aged , Birt-Hogg-Dube Syndrome/diagnosis , Humans , Male , Neoplasm Recurrence, Local/diagnosis , Neoplasms, Multiple Primary/diagnosis , Skin Neoplasms/diagnosisABSTRACT
INTRODUCTION: Heart manifestations of Churg-Strauss syndrome (CSS) are varied. In the early stages of the disease, it is difficult to distinguish between lesions that are specific to CSS and those of other etiologies. The aim of our work was to compare the characteristics of patients with heart manifestations linked or not to Churg-Strauss syndrome. MATERIAL AND METHODS: We recorded all clinical symptoms of patients with CSS hospitalized between 1998 and 2008 in Burgundy, France, and determined the possible relationships between heart symptoms and CSS. RESULTS: From a cohort of 31 patients, we found 20 with heart lesions. When heart lesions were present, we noted fewer initial symptoms of digestive disorders (p<0.05), lower levels of lung infiltrates and fewer anti-MPO pANCA (p<0.05). Heart lesions were linked to CSS in 75% of cases. Their patients were thus younger than those in the other cardiac patients (p<0.05), were more likely to have clinical manifestations of heart involvement at diagnosis, were less likely to have lung infiltrates on the X-ray at diagnosis and during flare-ups and less likely to have lung abnormalities on X-rays during flare-ups (p<0.05) and higher level of leucocytes and eosinophils at diagnosis. CONCLUSION: Heart lesions directly attributable to CSS are frequent, severe and probably underestimated. A specific physiopathology that is not mediated by ANCA seems to be involved in the genesis of CSS-related heart lesions.
Subject(s)
Churg-Strauss Syndrome/pathology , Myocardium/pathology , Age Factors , Aged , Aged, 80 and over , Churg-Strauss Syndrome/epidemiology , France/epidemiology , Heart/physiopathology , Humans , Incidence , Lung/pathology , Middle Aged , Odds Ratio , Prevalence , Retrospective Studies , Risk FactorsABSTRACT
Epidermolysis bullosa acquisita is a rare entity belonging to the auto-immune cutaneous blistering disorders of the dermo-epidermal junction. Clinical manifestations are generally cutaneous including the development of sub-epidermal blisters. Mucosal manifestations should be systematically looking for, but laryngeal involvement remains uncommon. We report an 81-year-old woman who presented with dysphagia, dyspnea and dysphonia as the presenting features of laryngeal involvement of an epidermolysis bullosa acquisita. This is the tenth reported case in the literature. We describe our diagnostic approach and the therapeutic management, comparing them with the literature.
Subject(s)
Epidermolysis Bullosa Acquisita/complications , Epidermolysis Bullosa Acquisita/pathology , Laryngitis/etiology , Laryngitis/pathology , Acute Disease , Aged, 80 and over , Anti-Infective Agents/therapeutic use , Dapsone/therapeutic use , Deglutition Disorders/etiology , Drug Therapy, Combination , Dysphonia/etiology , Dyspnea/etiology , Epidermolysis Bullosa Acquisita/diagnosis , Epidermolysis Bullosa Acquisita/drug therapy , Female , Glucocorticoids/therapeutic use , Humans , Laryngitis/complications , Laryngitis/diagnosis , Laryngitis/drug therapy , Prednisone/therapeutic use , Treatment OutcomeABSTRACT
Interferon-beta (IFN-beta) therapy can trigger immune adverse effects. We report a patient, who was treated for multiple sclerosis (MS) by IFN-beta during 3 years before developing a severe colitis due to Crohn's disease (CD). Neurological manifestations may occur in CD but rarely mimic MS. This report's points highlight the possible association of MS and CD, where IFN-beta therapy probably unmasked CD. Indeed, similar cases of CD flares have been reported after IFN-alpha therapy for chronic C hepatitis.
Subject(s)
Crohn Disease/chemically induced , Immunologic Factors/adverse effects , Interferon-beta/adverse effects , Multiple Sclerosis/drug therapy , Crohn Disease/diagnosis , Female , Hepatitis C, Chronic/drug therapy , Humans , Injections, Subcutaneous , Interferon-beta/administration & dosage , Magnetic Resonance Imaging , Middle Aged , Multiple Sclerosis/diagnosis , Time FactorsABSTRACT
Carcinomatous meningitis (CM) occurs in 3 to 8% of cancer patients. Patients present with a focal symptom, and multifocal signs are often found following neurological examination. The gold standard for diagnosis remains the demonstration of carcinomatous cells in the cerebrospinal fluid on cytopathological examination. Despite the poor prognosis, palliative treatment could improve quality of life and, in some cases, overall survival. We report on a patient who presented with vertigo, tinnitus and left-sided hearing loss followed by progressive diffuse facial nerve paralysis. Lumbar cerebrospinal fluid confirmed the diagnosis of CM. However, no primary tumor was discovered, even after multiple invasive investigations. This is the first reported case in the English-language medical literature of CM resulting from a carcinoma of unknown primary origin.
ABSTRACT
Diffuse and abundant sweating in a middle age patient evolving for several weeks should raise suspicion of malignant lymphoma and infectious or neuroendocrine disorders before considering a drug origin. We report a patient who presented with severe and invalidating excessive sweating related to hydromorphone therapy for vertebral pain. Amongst their many reported side-effects, excessive sweating disappearing with discontinuation of the drug have been reported with some opiates.
Subject(s)
Analgesics, Opioid/adverse effects , Hydromorphone/adverse effects , Hyperhidrosis/chemically induced , Aged , Analgesics, Opioid/administration & dosage , Back Pain/drug therapy , Humans , Hydromorphone/administration & dosage , MaleABSTRACT
INTRODUCTION: Celiac disease is a common condition with a prevalence of about 1%. Clinical extradigestive presentations are various and stroke can be one of the neurological manifestations. EXEGESIS: Two cases of stroke occurring in young adults are described, leading to the diagnosis of celiac disease. Hyperhomocysteinemia or cerebral arterial vasculopathy in one case and antiphospholipid syndrome in the other case are thought to be involved in the pathogenesis of stroke during celiac disease. CONCLUSION: The possible presence of celiac disease should be discussed in unexplained young adult stroke.
Subject(s)
Celiac Disease/diagnosis , Stroke/etiology , Adult , Antiphospholipid Syndrome/complications , Celiac Disease/complications , Celiac Disease/therapy , Cerebral Arterial Diseases/complications , Female , Humans , Hyperhomocysteinemia/complications , Hyperhomocysteinemia/therapy , Magnetic Resonance Angiography , Magnetic Resonance Imaging , Male , Middle Cerebral Artery , Stroke/diagnosisABSTRACT
We report the case of a 41-year-old woman, non-smoker, without cardiovascular risk, hospitalised for acute ST elevation myocardial infarction. The coronarography showed no significant coronary stenosis and a methylergometrine test performed was positive. After the discovery of a moderate chronic hypereosinophilia, a review of the literature about eosinophile and coronary pathology was realised in order to make a link between hypereosinophilia and coronary spasm.
Subject(s)
Coronary Vasospasm/etiology , Eosinophilia/complications , Adult , Coronary Vasospasm/complications , Female , Humans , Myocardial Infarction/etiologyABSTRACT
INTRODUCTION: Anterior scleritis is defined as an inflammation of the sclera, located anteriorly to the equator of the eye. Cotrimoxazole is an antibiotic with an immunomodulatory action. EXEGESIS: In case of idiopathic anterior scleritis or scleritis associated with autoimmune diseases, immunosuppressive treatment is often required. We report on six patients with anterior idiopathic scleritis non sensitive to local treatment where cotrimoxazole improved or cured the symptoms. CONCLUSION: Cotrimoxazole seems to be an interesting therapeutic treatment in non threatening anterior scleritis.